Polyarteritis nodosa

Polyarteritis nodosa (or periarteritis nodosa) is a vasculitis of medium-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Meier disease.^[1]

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Causes and risk factors

Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues. It occurs when certain immune cells attack the affected arteries. One hypothesis is that this condition is caused by antibodies against HBV, via a type III hypersensitivity reaction.

Incidence

The condition affects adults more frequently than children. It damages the tissues supplied by the affected arteries because they don't receive enough oxygen and nourishment without a proper blood supply. Polyarteritis nodosa is more common in people with hepatitis B infection.

Symptoms

In this disease, symptoms result from damage to affected organs, often the skin, heart, kidneys, and nervous system.

Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps.

Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal failure.

Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart (pericarditis).

• Fatigue
• Weakness
• Fever
• Abdominal pain
• Decreased appetite
• Unintentional weight loss
• Muscle aches
• Joint aches

Signs and tests

There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is generally based upon the physical examination and a few laboratory studies that help to confirm the diagnosis:

• CBC (may demonstrate an elevated white blood count)
• ESR (elevated)
• Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitis or leukocytoclastic angiitis.
• Tissue biopsy (reveals inflammation in small arteries, called arteritis)
• Elevated c reactive protein

A patient is said to have polyarteritis nodosa if he or she has 3 of the 10 following signs:

  1. Weight loss greater than/equal to 4 kg.
  2. Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso).
  3. Testicular pain or tenderness. (occasionally, a site biopsied for diagnosis).
  4. Muscle pain, weakness, or leg tenderness.
  5. Nerve disease (either single or multiple).
  6. Diastolic blood pressure greater than 90mmHg (high blood pressure).
  7. Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinine greater than 1.5 mg/dl).
  8. Hepatitis B virus tests positive (for surface antigen or antibody).
  9. Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation.
 10. Biopsy of tissue showing the arteritis (typically inflamed arteries).[2]

Treatment and Prognosis

Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. Without treatment, the outlook is poor.

Potassium citrate was able to prevent arterial lesions in sodium chloride loaded hypertensive dahl rats without lowering the blood pressure. Potassium chloride had a somewhat lesser protective affect ^[3].

Complications

• Stroke
• Kidney failure
• Heart attack
• Intestinal necrosis and perforation

Prevention

This disease cannot be currently prevented, but early treatment can prevent some damage and symptoms.

References

1. ^ synd/764 at Who Named It
2. ^ Shiel, Jr., William C, http://www.medicinenet.com/polyarteritis_nodosa/article.htm
3. ^ Tobian L MacNeill D Johnson MA Gagul MC & Iwai J 1984 Potassium protection against lesions of the renal tubules, arteries, and nephron loss in salt loaded hypertensive dahl S rats. Hypertension, supplement I 6: 170-176.

acquired deformities of fingers and toes (Boutonniere deformity, Bunion, Hallux rigidus, Hallux varus, Hammer toe) - other acquired deformities of limbs (Valgus deformity, Varus deformity, Wrist drop, Foot drop, Flat feet, Club foot, Unequal leg length, Winged scapula)

patella (Luxating patella, Chondromalacia patellae)

synovium and tendon: Synovitis - Tenosynovitis (Stenosing tenosynovitis, Trigger finger, DeQuervain's syndrome)

bursitis (Olecranon, Prepatellar, Trochanteric)

fibroblastic (Dupuytren's contracture, Plantar fasciitis, Nodular fasciitis, Necrotizing fasciitis, Fasciitis, Fibromatosis)

enthesopathies (Iliotibial band syndrome, Achilles tendinitis, Patellar tendinitis, Golfer's elbow, Tennis elbow, Metatarsalgia, Bone spur, Tendinitis)